连枷臂综合征颈区上运动神经元损害的检测

Assessment of the function of cervical spinal upper motor neuron in patients with frail arm syndrome

目的 对连枷臂综合征（FAS）患者进行体检、胸肌腱反射定量化检测和三重刺激技术（TST）检测,探寻其颈区上运动神经元（UMN）损害的证据.方法 分析2013年6月至2016年6月于北京大学第三医院神经内科就诊的FAS患者67例,观察其诊断时和随访3、6、9、12个月时临床体检、神经电生理检测结果,主要记录体检时的锥体束征、胸肌腱反射定量化检测和TST检测结果,判断其颈区UMN功能状况.结果 67例FAS患者诊断时,双上肢近端无力61例,右上肢近端无力4例,左上肢近端无力2例.上肢腱反射增强25例,减弱20例,消失22例.胸肌腱反射活跃或亢进66例,减弱1例,颈区UMN受累评分（2.0±0.5）分,颈区下运动神经元受累评分（2.0±0.2）分.TST检测结果表明TST检测/TST对照波幅比为78.31％ ±6.52％.胸肌腱反射定量化检测结果显示,胸肌腱反射的潜伏期为（7.80±1.22） ms,波幅为（1.23±0.14） mV.随访研究发现上肢腱反射逐渐减弱,UMN评分逐渐减低,下运动神经元评分逐渐增高,TST检测/TST对照波幅比逐渐下降,胸肌腱反射定量化检测潜伏期变化不明显.结论 FAS患者存在颈区UMN损害,随着病程进展,锥体束征常常被肌萎缩掩盖.

Objective To investigate cervical spinal upper motor neuron （UMN） dysfunction in patients with frail arm syndrome （FAS） by physical examination,triple stimulation technique （TST） and pectoralis tendon reflex tests.Methods Sixty-seven FAS patients,coming from Peking University Third Hospital from June 2013 to June 2016,underwent physical examination and routine electrophysiological tests.The pyramid signs,the results of pectoralis tendon reflex and TST were collected to estimate the function of cervical spinal UMN.Results On the time of diagnosis,weakness of bilateral proximal upper limbs was found in 61 patients,while weakness of unilateral proximal upper limb was found in six patients.There were 25 patients with tendon hyperreflexia,20 patients with tendon hyporeflexia and 22 patients with tendon areflexia.All the patients were pectoral muscle tendon hyperreflexia except one.UMN score of cervical region was 2.0 ± 0.5.Lower motor neuron score of cervical region was 2.0 ± O.2.The amplitude ratio of TSTtest/TSTcontrol was 78.31% ± 6.52%.The latency and amplitude of quantitative detection of pectoralis tendon reflex was （7.80 ± 1.22） ms and （1.23 ± 0.14） mV,respectively.In the follow-up study,the tendon reflexes and the UMN score declined,the amplitude ratio of TSTtest/TSTcontrol decreased,while the lower motor neuron score increased and the latency of quantitative detection of pectoralis tendon reflex remained almost unchanged.Conclusion The results showed that there was cervical spinal UMN dysfunction in patients with FAS,and the pyramid signs were often concealed by muscle atrophy with progression of the disease.